BioMed Central Page 1 of 3 (page number not for citation purposes) World Journal of Surgical Oncology Open Access Case report A giant hemolymphangioma of the pancreas in a 20-year-old girl: a report of one case and review of the literature Li-Feng Sun 1 , Hui-Lin Ye 2 , Qi-Yan Zhou 3 , Ke-Feng Ding* 1 , Pei-Lin Qiu 1 , Yong-Chuan Deng 1 , Shu-Zhan Zhang 1 and Shu Zheng 1 Address: 1 Department of Surgical Oncology, the Second Affiliated Hospital, College of Medicine, Zhejinag University, Hangzhou Zhejiang 310009, PR China, 2 People's Hospital of Songyang, Songyang, Zhejiang 323400, PR China and 3 Zhejiang Qingchun Hospital, Hangzhou, Zhejiang 310013, PR China Email: Li-Feng Sun - sunlifeng@medmail.com.cn; Hui-Lin Ye - yehuilin@yahoo.com.cn; Qi-Yan Zhou - zhouqiyan@medmail.com.cn; Ke- Feng Ding* - sun_zju@yahoo.com.cn; Pei-Lin Qiu - qiupeilin@fa.zju.edn.cn; Yong-Chuan Deng - dengyongchuan@fa.zju.edu.cn; Shu- Zhan Zhang - zhangscy@163.net; Shu Zheng - zhengshu@zju.edu.cn * Corresponding author Abstract Background: Hemolymphangioma of the pancreas is a very rare benign tumor. There were only six reports of this disease until December 2008. Herein, we report a case of giant hemolymphangioma of the pancreas in a 20-year-old girl. Case presentation: We describe a 20-year-old girl who presented with a mass in abdominal cavity and epigastric discomfort about a week. Physical examination showed a great abdominal mass. Abdominal computed tomography showed extrinsic duodenal compression due to a large retroperitoneal tumor possibly arising from pancreas. The tumor enucleation was performed and a diagnosis of hemolymphangioma of the pancreas was made. The patient had a complication of chylous leakage, which was successfully managed. The patient is alive and well, after 26 months of follow-up, with no complaints or recurrence. Conclusion: From this case and literature, we can conclude that hemolymphangioma of the pancreas in adult is a rare benign tumor, and accurate diagnosis can not be preoperatively established. Tumor resection should be performed whenever possible. The risk of recurrence seems very low. Background Hemolymphangioma of the pancreas is a rare disease and basically benign cystic tumor. There were only six reports of this tumor of the pancreas until December 2008 (PubMed) [1-6]. Cystic tumors of the pancreas account for approximately 10% to 15% of cystic lesions of the pan- creas. Vascular tumors of the pancreas are cystic tumors accounting for 0.1% of all pancreatic tumors[7]. Major symptoms in this hemolymphangioma are a mass in abdominal cavity and epigastric discomfort associated with the enlarged tumor. We present a large hemolym- phangioma of the pancreas in a 20-year-old girl with a review of the literature. Case presentation The patient was a 20-year-old girl who complained of a mass in abdominal cavity and epigastric discomfort about a week. She was a college student. On admission she was Published: 18 March 2009 World Journal of Surgical Oncology 2009, 7:31 doi:10.1186/1477-7819-7-31 Received: 25 December 2008 Accepted: 18 March 2009 This article is available from: http://www.wjso.com/content/7/1/31 © 2009 Sun et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0 ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. World Journal of Surgical Oncology 2009, 7:31 http://www.wjso.com/content/7/1/31 Page 2 of 3 (page number not for citation purposes) well, not vomit, stomachache and icteric. Physical exami- nation showed a large abdominal mass. Abdominal com- puted tomography showed extrinsic duodenal compression due to a large retroperitoneal tumor possibly arising from pancreas, which had polycystic structure and partial blood flow (Figure 1). No abnormalities were revealed in laboratory data including tumor markers such as CEA and CA19-9. At laparotomy there was a black poly- cystic, retroperitoneal tumor extending from coeliac axis to the origin of the head of pancreas. The tumor infiltrated the transverse mesocolon, greater omentum, and tightly adhered to duodenum and superior mesenteric artery. Along the surface of the duodenum and pancreas, tumor (including partial transverse mesocolon and greater omentum) excision was performed. Pancreatoduodenec- tomy was not performed. Macroscopically, the mass measured 18 × 16 × 12.5 cm. It was nodular, and soft in consistency. The tumors were multiloculated cystic masses full of bloody fluid. Micro- scopically(Figure 2), the tumor showed a soft tissue mass consisted of lymphatic and blood vessels with polycystic spaces. There was infiltration of the stroma by lym- phocytes. The definitive histological diagnosis was hemo- lymphangioma of the pancreas. After operation, the patient had a complication of chylous leakage, which was successfully managed. She was cured and dischaged after 20 days after surgery. After 26 months of follow-up by computed tomography and Ultrasonogra- phy, there was no complaints or recurrence. Discussion Intra-abdominal hemolymphangioma is very rare; rarer still is the involvement of pancreas. On a review of pub- lished work (online PubMed search) till December 2008 (Pubmed), we found only 6 case reports [1-6]. Their char- acteristics were showed below (Table 1). This tumor is considered a congenital malformation of the vascular sys- tem. The formation of this tumor may be explained by obstruction of the venolymphatic communication between dysembrioplastic vascular tissue and the systemic circulation[5]. These lesions may arise from the pancreatic parenchyma[5]. Hemolymphangioma of pancreas are usually large lesions with a diameter of larger than 10 cm, and the commonest site is the head of pancreas. Generally, they are large masses with thin wall having multiple thin septa with var- ying size cystic cavities containing fluid similar to hemor- rhagic and rarely of clear lymphatic nature. Microscopically, the tumor consists of abnormal lym- phatic and blood vessels with polycystic spaces. These cysts have connective septa covered by endothelium. This tumor may be asymptomatic for a long time. Abdom- inal pain and awareness of abdominal mass are the most common symptoms. Other infrequent symptoms such as vomiting and nausea are caused by occupied tumor. This tumor is commonly a benign disease and has no invasion ability. But in the 6th case reported by a Japanese group[6], the chief complaint was severe anemia caused by duodenal bleeding because the hemolymphangioma of the pancreas invaded to the duodenum. This symptom is extremely rare. In our case, the chief symptoms were a Abdominal Computed tomography demonstrating a large tumour with partial blood flow(arrow) in abdominal cavityFigure 1 Abdominal Computed tomography demonstrating a large tumour with partial blood flow(arrow) in abdominal cavity. Low-power review showing a soft tissue mass consists of lymphatic and blood vessels with polycystic spaces (H & E ×100)Figure 2 Low-power review showing a soft tissue mass con- sists of lymphatic and blood vessels with polycystic spaces (H & E ×100). World Journal of Surgical Oncology 2009, 7:31 http://www.wjso.com/content/7/1/31 Page 3 of 3 (page number not for citation purposes) giant mass in abdominal cavity and epigastric discomfort. At laparotomy tumor infiltrated the transverse mesocolon and greater omentum, and tightly adhered to the duode- num and superior mesenteric artery. Generally, this dis- ease is benign, but it is possible that this tumor invaded other organs like our case and Japanese case. The clinical diagnosis of hemolymphangioma of pancreas is not often due to its rarity and the absence of clinical expression. Laboratory tests are frequently normal although the case reported by Banchini [4] had a slight increase in alkaline phosphatase and gamma-glutamyl transferase. Serum carcinoembryonic antigen (CEA) and CA19-9 are within normal limits. Imaging techniques such as ultrasonography, Abdominal Computed tomogra- phy, and magnetic resonance imaging may be used to assess, make a clinical diagnosis and for follow-up. The impossibility to preoperatively define the histological type of the tumor explains the difficulties to reach a cor- rect differential diagnosis. Clinical differential diagnoses includes pseudocyst, lymphangioma, serous from muci- nous tumors, sarcoma, enteric duplication cyst, and cystic tumor not otherwise specified. The final diagnosis is based on a combination of clinical, radiological, and his- topathological findings. Surgery including local resection of this tumor is a defini- tive modality. Two operative attitudes are possible: the tumoral enucleation and the partial pancreatectomies. Hemolymphangioma of the pancreas is commonly a benign disease and has no invasion ability. Local resec- tion is necessary. But in the Japanese case, pancreatoduo- denectomy was performed because the tumor invaded to the duodenum to cause the duodenal bleeding. In addi- tion, pancreatoduodenoctomy is performed for suspicion of malignancy. All cases in the literature had good prog- nosis as did our case. The risk of recurrence or metastasis seems very low, but careful follow-up is necessary. Herein, we reported a case of hemolymphangioma of the pancreas head with a large size in a 20-year-old girl. The wide infiltration and adherence of adjacent organs and tis- sues was an important feature of the present case. Conclusion From this case and literature, we can conclude that hemo- lymphangioma of the pancreas in adult is a rare benign tumor, and accurate diagnosis can not be preoperatively established. Tumor resection should be performed when- ever possible. The risk of recurrence seems very low. Despite its low frequency, this disease should be consid- ered when a multiloculated cystic masses in abdominal cavity is seen. Consent Written consent was obtained from the patient or their relative for publication of study. Competing interests The authors declare that they have no competing interests. Authors' contributions LFS and HLY designed the study, performed picture acqui- sition and drafted part of the manuscript. PLQ and KFD performed the surgery, carried out data acquisition and drafted part of the manuscript. All authors participated in the editing and have read and approved the final manu- script. References 1. Couinaud , Jouan , Prot , Chalut , Schneiter : [Hemolymphangi- oma of the head of the pancreas]. Mem Acad Chir (Paris) 1966, 92:152-155. 2. Couinaud C, Jouan , Prot , Chalut , Favre , Schneiter : [A rare tumor of the head of the pancreas. (Hemolymphangioma weighing 1,500 kg.)]. Presse Med 1967, 75:1955-1956. 3. Montete P, Marmuse JP, Claude R, Charleux H: [Hemolymphangi- oma of the pancreas]. J Chir (Paris) 1985, 122:659-663. 4. Banchini E, Bonati L, Villani LG: [A case of hemolymphangioma of the pancreas]. Minerva Chir 1987, 42:807-813. 5. Balderramo DC, Di Tada C, de Ditter AB, Mondino JC: Hemolym- phangioma of the pancreas: case report and review of the lit- erature. Pancreas 2003, 27:197-199. 6. Toyoki Y, Hakamada K, Narumi S, Nara M, Kudoh D, Ishido K, Sasaki M: A case of invasive hemolymphangioma of the pancreas. World J Gastroenterol 2008, 14:2932-2934. 7. Le Borgne J, de Calan L, Partensky C: Cystadenomas and cystad- enocarcinomas of the pancreas: a multiinstitutional retro- spective study of 398 cases. French Surgical Association. Ann Surg 1999, 230:152-161. Table 1: Characteristics of six patients with hemolymphangioma of the pancreas[5] Case No. Age(y) sex Site of pancreas Size/weight Treatment Prognosis 1 68 F head -/1450 g PD No recurrence 2 66 F head -/1500 g PD+PG No recurrence 3 31 F Body/tail 14 cm/- BTP No recurrence 4 67 F head 15 cm/- PD No recurrence 5[5] 53 F head 4*3 cm/- PD No recurrence 6[6] 53 M head >6 cm/- PD No recurrence PD, pancreatoduodenectomy; PG, partial gastrectomy; BTP, body and tail pancreatectomy. . reported a case of hemolymphangioma of the pancreas head with a large size in a 20-year-old girl. The wide infiltration and adherence of adjacent organs and tis- sues was an important feature of the. enucleation was performed and a diagnosis of hemolymphangioma of the pancreas was made. The patient had a complication of chylous leakage, which was successfully managed. The patient is alive and. hemolymphangioma are a mass in abdominal cavity and epigastric discomfort associated with the enlarged tumor. We present a large hemolym- phangioma of the pancreas in a 20-year-old girl with a review